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. 2025 Jun 25.
doi: 10.1245/s10434-025-17364-z. Online ahead of print.

Consensus Guideline for the Management of Patients with Appendiceal Tumors, Part 2: Appendiceal Tumors with Peritoneal Involvement

Elizabeth L Godfrey  1 , Forest Mahoney  2 , Varun V Bansal  1 , David G Su  1 , David N Hanna  3 , Felipe Lopez-Ramirez  4 , Ekaterina Baron  5 , Kiran K Turaga  6 , Al B Benson 3rd  7 , Namrata Setia  8 , Joshua H Winer  9 , Craig G Gunderson  1   10   11 , Rupen Shah  12 , Deepa R Magge  3 , Ian Solsky  13 , Cathy Eng  14 , Oliver S Eng  15 , Ardaman Shergill  16 , John Paul Shen  17 , Joseph Misdraji  18 , Michael B Foote  19 , Wenyi Luo  18 ; Peritoneal Surface Malignancies (PSM) Consortium Group
Collaborators, Affiliations

Consensus Guideline for the Management of Patients with Appendiceal Tumors, Part 2: Appendiceal Tumors with Peritoneal Involvement

Elizabeth L Godfrey et al. Ann Surg Oncol. .

Abstract

Background: Appendiceal tumors comprise a heterogeneous group of tumors that frequently disseminate to the peritoneum. Management of appendiceal tumors is lacking high quality data given their rarity and heterogeneity. In general, appendiceal tumor treatment is extrapolated in part from colorectal cancer or pooled studies, without definitive evidence of disease-specific benefit. Many practices are controversial and vary widely between institutions. A national consensus update of best management practices for appendiceal malignancies was performed to better standardize care. Herein the authors present recommendations for the management of appendiceal tumors with peritoneal involvement.

Methods: As previously described, modified Delphi consensus was performed to update the previous 2018 Chicago consensus guideline. Recommendations were supported by using rapid systematic reviews of key issues in surgical and systemic therapy. Key pathology concepts and recommendations were synthesized in collaboration with content experts.

Results: A consensus-based pathway was generated for any type of non-neuroendocrine appendiceal tumor with peritoneal involvement. The first round of Delphi consensus included 138 participants, of whom 133 (96%) participated in the second round, and greater than 90% consensus was achieved for all pathway blocks. Key items include recommending evaluation for cytoreduction to most patients with low-grade peritoneal disease who are surgical candidates, and to many patients with high-grade disease, as well as timing of systemic chemotherapy and surveillance protocols. Common pitfalls in pathologic classification and their clinical implications are also presented.

Conclusion: These consensus recommendations provide guidance regarding the management of appendiceal tumors with peritoneal involvement, including a review of current evidence in the management of recurrent and unresectable disease.

Keywords: Appendiceal malignancies; Cytoreductive surgical procedures; Guidelines; Peritoneal neoplasms; Peritoneal surface malignancies.

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Conflict of interest statement

Disclosure: Al B. Benson, III, MD5: Dr. Benson has grants and contracts with the following entities in which funds are provided directly to Northwestern University: Infinity Pharmaceuticals, Merck Sharp & Dohme Taiho Pharmaceutical, Bristol-Myers Squibb –DMC, Celgene, Rafael Pharmaceuticals, MedImmune, Xencor, Astellas Pharma – DMC, Amgen, Syncore – DMC, Tyme Inc – DMC, ITM Solucin GmbH, PANC003 – Rafael Pharmaceuticals, Inc., RM-110 – Elevar Therapeutics, Inc., STP-ST-01 - ST Pharm Co., Ltd., and Mirati Therapeutics. He has personal grant and contract relationships with Elevar Therapeutics, Inc, ITM Solucin, Janssen Merck Sharp and Dohme LLC, Pfizer, ITM Samsung Bioepsis, National Cancer Institute Lead Academic Participating Site ST Pharm CO. Ltd, The Nathan Cummings Foundation, and Xenor. He receives support for meetings and travel associated with NCCN panel meetings, ACCC meetings, and Fight Colorectal Cancer Advocacy. He participates on advisory and DSMBs for the following: BMS, Astellas, Apexigen, PrecisCA, Array (Pfizer); Novartis DMC, Amgen, Terumo, Mirati, GSK, Bayer, Aveo DMC, Gusto, Boehringer Ingelheim, Abbvie, Artemida, White Swan, Harborside Press, Patient Resources, LLC, Axis Medical Education, Envision Communications, Trialcard Incorp, Tempus Labs, Inc, Aptitude Health, Therabionic, Aptitude Health, Clarivate Analytics US LLC, Tukysa, Natera, Janssen, AIM Immuno Tech, Nuvation Bio, Xencor, Janssen, Natera, Xenor, Boston Scientific, Tukysa, and Cardiff Oncology. He participates in the following unpaid leadership or fiduciary roles: Board of Directors NCCN and NCCN Foundation, Patient Advocate Foundation (PAF), National Patient Advocate Foundation (NPAF), Scientific Advisor for Fight Colorectal Cancer. Namrata Setia, MD7: Dr. Setia is on the Speaker Bureau of Astellas and also has served as a consultant for Bristol Myers Squibb in the past 2 years. Oliver S. Eng, MD13: Dr. Eng receives speaker fees from Tempus Labs, Inc. Ardaman Shergill, MD, MSPH14: Dr. Shergill is on the advisory board for Pfizer, Guardant, and Natera. She has received travel, registration, and accommodation support for presenting at AACR from Takeda. She is also the recipient of research related funding from the following entities in which funds are provided directly to University of Chicago: Hutchison MediPharma, Merck, Verastem Oncology, Turning Point Therapeutics, Gritstone, Bolt Therapeutics, BMS, Pfizer, Astellas, Oncologie, Macogenics, Seattle Genetics, Amgen, Daiichi, Lilly, Jacobio, and Takeda. John Paul Shen, MD15: reported receiving personal fees from Nadeno Nanoscience and Engine Bioscience; receiving grants from Celsius Therapeutics and BostonGene outside the submitted work; holding a patent for small molecule GNAS inhibitors; and serving on the Medical Advisory Board for Appendix Cancer Pseudomyxoma Peritonei Research Foundation (unpaid). Other authors have no relevant financial disclosures. Ethics Approval and Consent to Participate: Not applicable. Consent for Publication: Not applicable. Declaration of Generative AI and AI-Assisted Technologies in the Writing Process: No AI or AI-assisted technologies were used during the development of this manuscript.

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